Results 11 to 20 of about 26,122 (235)

Nucleoside-Modified mRNA Encoding Alpha-Galactosidase A Ameliorates Fabry Disease Phenotypes in Human IPSC-Derived Cardiomyocytes. [PDF]

Adv Sci (Weinh)
Human iPSC‐derived Fabry cardiomyocytes exhibited broad transcriptional dysregulation, apoptosis, mitochondrial dysfunction, impaired reactive oxygen species handling, altered contractility, and abnormal calcium transient decay, potentially mediated by phospholamban hyperphosphorylation.
Juchem M, Oehlsen L, Ersoy S, Ye JL, Weber N, Mohr E, Agyapong W, Liu J, Fuchs M, Xiao K, Jahn C, Eggenschwiler R, Cantz T, Beimdiek J, Buettner FFR, Kraft T, Hegermann J, Knorr IJ, Ernst L, Feldbrügge L, Puffert R, Schmidt K, Bär C, Hoepfner J, Thum T.   +24 more
europepmc   +2 more sources

Lipidomics of Glycosphingolipids [PDF]

Metabolites, 2012
Glycosphingolipids (GSLs) contain one or more sugars that are attached to a sphingolipid moiety, usually to a ceramide, but in rare cases also to a sphingoid base. A large structural heterogeneity results from differences in number, identity, linkage, and anomeric configuration of the carbohydrate residues, and also from structural differences within ...
Farwanah, Hany, Kolter, Thomas
openaire   +4 more sources

Glycosphingolipid-Glycan Signatures of Acute Myeloid Leukemia Cell Lines Reflect Hematopoietic Differentiation

, 2022
Aberrant expression of certain glycosphingolipids (GSLs) is associated with the differentiation of acute myeloid leukemia (AML) cells. However, the expression patterns of GSLs in AML are still poorly explored because of their complexity, the presence of ...
Marieke Griffioen (119817), Guinevere S.M. Lageveen-Kammeijer, Guinevere S.M. Lageveen-Kammeijer (9451317), Robbert M. Spaapen, Constantin Blöchl (12123532), Oleg A. Mayboroda (340333), Constantin Blöchl, Manfred Wuhrer, Antonius A. de Waard, Di Wang (329735), Robbert M. Spaapen (6270995), Christian G. Huber, Oleg A. Mayboroda, Marieke Griffioen, Manfred Wuhrer (200842), Tao Zhang, Tao Zhang (43681), Antonius A. de Waard (6270992), Katarina Madunić, Katarina Madunić (10724034), Di Wang, Christian G. Huber (355980)   +21 more
core   +2 more sources

Genome analysis of the glycosphingolipid-producing green alga tetraselmis sp. NKG400013. [PDF]

DNA Res
Microalgae are gaining attention as sustainable resources for the production of valuable compounds, including biofuels, pigments, and bioactive metabolites.
Yasui R, Hosaka A, Ogata N, Ogawa T, Yohda M, Arakaki A, Kataoka K, Tanaka T.   +7 more
europepmc   +2 more sources

Disialoganglioside GD2-Targeted Near-Infrared Photoimmunotherapy (NIR-PIT) in Tumors of Neuroectodermal Origin

Pharmaceutics, 2022
Disialoganglioside (GD2) is a subtype of glycolipids that is highly expressed in tumors of neuroectodermal origins, such as neuroblastoma and osteosarcoma.
Fuyuki F. Inagaki, Takuya Kato, Aki Furusawa, Ryuhei Okada, Hiroaki Wakiyama, Hideyuki Furumoto, Shuhei Okuyama, Peter L. Choyke, Hisataka Kobayashi   +8 more
doaj   +1 more source

Glycosphingolipid Functions [PDF]

Cold Spring Harbor Perspectives in Biology, 2011
The combination of carbohydrate and lipid generates unusual molecules in which the two distinctive halves of the glycoconjugate influence the function of each other. Membrane glycolipids can act as primary receptors for carbohydrate binding proteins to mediate transmembrane signaling despite restriction to the outer bilayer leaflet.
openaire   +3 more sources

An Inducible Mouse Model of Late Onset Tay–Sachs Disease

Neurobiology of Disease, 2002
Mouse models of the GM2 gangliosidoses, Tay–Sachs and Sandhoff disease, are null for the hexosaminidase α and β subunits respectively. The Sandhoff (Hexb−/−) mouse has severe neurological disease and mimics the human infantile onset variant. However, the
Mylvaganam Jeyakumar, David Smith, Elena Eliott-Smith, Mario Cortina-Borja, Gabriele Reinkensmeier, Terry D. Butters, Thorsten Lemm, Konrad Sandhoff, V.Hugh Perry, Raymond A. Dwek, Frances M. Platt   +10 more
doaj   +1 more source

Effect of Glycosphingolipids on Osteoclastogenesis and Osteolytic Bone Diseases

Frontiers in Endocrinology, 2012
Alterations in glycosphingolipid production results in lysosomal storage disorders associated with neurodegenerative changes. In Gauchers disease, the patients also develop osteoporosis that is ameliorated upon treatment for the underlying defect in ...
Adel eErsek, Anastasios eKaradimitris, Nicole eHorwood   +2 more
doaj   +1 more source

The role of neuraminidase 1 (NEU1) in cytokine release by primary mouse mesangial cells and disease outcomes in murine lupus nephritis

Autoimmunity, 2021
The importance of altered glycosphingolipid (GSL) metabolism is increasingly gaining attention as a characteristic of multiple chronic kidney diseases. Previously, we reported elevated levels of GSLs and neuraminidase (NEU) enzyme activity/expression in ...
Jessalyn Rodgers, Kamala Sundararaj, Evelyn Bruner, Bethany Wolf, Tamara K. Nowling   +4 more
doaj   +1 more source

Glycosphingolipid Levels in Urine Extracellular Vesicles Enhance Prediction of Therapeutic Response in Lupus Nephritis

Metabolites, 2022
The development of nephritis increases the risk of morbidity and mortality in systemic lupus erythematosus (SLE) patients. While standard induction therapies, such as mycophenolate mofetil (MMF) induce clinical remission (i.e., complete response) in ...
Brian Troyer, Jessalyn Rodgers, Bethany J. Wolf, James C. Oates, Richard R. Drake, Tamara K. Nowling   +5 more
doaj   +1 more source